Distinctive syndromes of isolated intrahepatic bile duct paucity and an intact extrahepatic biliary tree.

Alagille syndrome (arteriohepatic dysplasia) is the most common syndrome incorporating intrahepatic bile duct paucity.
facial characteristics (broad forehead; deep-set, widely spaced eyes; long, straight nose; and an underdeveloped mandible). There may also be ocular abnormalities (posterior embryotoxon), cardiovascular abnormalities (usually peripheral pulmonic stenosis, sometimes tetralogy of Fallot), vertebral arch defects and failure of anterior vertebral arch fusion (butterfly vertebrae), and tubulointerstitial nephropathy. There are several forms of intrahepatic cholestasis due to defects in specific transport proteins involved in bile formation (Table 337-2). Byler disease, a severe form of progressive intrahepatic cholestasis (PFIC type 1), The disease is characterized by unique structural abnormalities in the bile canalicular membrane. Affected patients present with failure to thrive, steatorrhea, pruritus, rickets, and low -glutamyl transpeptidase levels. Cirrhosis gradually develops. The major clinical differentiation from Alagille syndrome is the absence of bile duct paucity and extrahepatic features.

PFIC type 1 (Byler disease) is mapped to chromosome 18q12. Affected patients have low serum levels of -glutamyltransferase, normal serum cholesterol, and high serum bile acid levels. A mutation in P-type membrane adenosine triphosphatase is the responsible mechanism in Amish families. PFIC type 2 is similar to Byler disease but is present in non-Amish families (Middle Eastern Europeans) and has a gene locus at chromosome 2q24. Mutations of BSEP gene, which codes for a bile canalicular adenosine triphosphate-dependent bile acid transporter, may be responsible. PFIC type 3 is characterized by high serum levels of -glutamyltransferase and histologically by portal bile duct inflammation and proliferation. There is complete absence of the multidrug resistance 3 P glycoprotein, with deficient translocation of phosphatidylcholine across the canalicular membrane. Mothers who are heterozygous for this gene are at risk for intrahepatic cholestasis of pregnancy. Aagenaes syndrome is a form of idiopathic familial intrahepatic cholestasis associated with lymphedema of the lower extremities. The relation between the liver disease and lymphedema is not understood and may be attributable to decreased hepatic lymph flow or hepatic lymphatic hypoplasia. Affected patients usually present with episodic cholestasis with elevation of serum aminotransferases, alkaline phosphatase, and bile acids. Between the episodes the patients are usually asymptomatic and the biochemical indices improve. The locus for Aagenaes syndrome has been mapped to a 6.6-cM interval on chromosome 15q. Zellweger (cerebrohepatorenal) syndrome is a rare autosomal recessive genetic disorder marked by progressive degeneration of the liver and kidneys (Chapter 75.2). The incidence is estimated to be 1/100,000 births; the disease is usually fatal within 6-12 mo. Affected infants have severe, generalized hypotonia and markedly impaired neurologic function with psychomotor retardation. Patients have an abnormal head shape and unusual facies, hepatomegaly, renal cortical cysts, stippled calcifications of the patellas and greater trochanter,

and ocular abnormalities. Hepatic cells on ultrastructural examination show an absence of peroxisomes. Neonatal iron storage disease (NISD) is a rapidly progressive disease characterized by increased iron deposition in the liver, heart, and endocrine organs without increased iron stores in the reticuloendothelial system. Patients have multiorgan failure and shortened survival. Familial cases have been reported and the disease is most likely transmitted in an autosomal recessive or codominant pattern. Laboratory findings include hypoglycemia, hyperbilirubinemia, hypoalbuminemia and profound hypoprothrombinemia. Serum aminotransferases may be high initially but normalize with the progression of the disease. The diagnosis is usually confirmed by buccal mucosal biopsy or magnetic resonance imaging demonstrating extrahepatic siderosis. The prognosis is poor; liver transplantation can be curative. Despite initially encouraging reports, the use of a combination of antioxidants and prostaglandin infusion with chelation may not uniformly improve outcome in the patients with NISD.

Defective bile acid biosynthesis has been postulated to be an

initiating or perpetuating factor in neonatal cholestatic disorders; the hypothesis is that inborn errors in bile acid biosynthesis lead to absence of normal trophic or choleretic primary bile acids and accumulation of primitive (hepatotoxic) metabolites. Inborn errors of bile acid biosynthesis is a recognizable cause of acute and chronic liver disease; early recognition allows institution of targeted bile acid replacement, which reverses the hepatic injury. Several specific defects have been described:

Deficiency of 4-3-oxosteroid-5 reductase, the fourth step in the pathway of cholesterol degradation to the primary bile acids is manifested as significant cholestasis and liver failure developing shortly after birth with coagulopathy and metabolic liver injury resembling tyrosinemia. Hepatic histology is characterized by lobular disarray with giant cells, pseudoacinar transformation, and canalicular bile stasis. Mass spectrometry is required to document increased urinary bile acid excretion and the predominance of oxo-hydroxy and oxo-dihydroxy cholenoic acids. Treatment with cholic acid and ursodeoxycholic acid is associated with normalization of biochemical, histologic, and clinical features. Deficiency of 3-hydroxy C27-steroid dehydrogenase (3-HSD) isomerase, the second step in bile acid biosynthesis, causes progressive familial intrahepatic cholestasis. Affected patients usually have jaundice with increased aminotransferase levels and hepatomegaly; -glutamyl transpeptidase levels and serum cholylglycine levels are normal. The histology is variable, ranging from giant cell hepatitis to chronic hepatitis. The diagnosis, suggested by mass spectrometry detection of C24 bile acids in urine, which retain the 3-hydroxy-5 structure, can be confirmed by determination of 3-HSD activity in cultured fibroblasts using 7-hydroxy-5 cholesterol as a substrate. Primary bile acid therapy, administered orally to downregulate cholesterol 7-hydroxylase activity, limit the production of 3-hydroxy-5 bile acids, and facilitate hepatic clearance, has been effective in reversing hepatic injury. Another metabolic defect in bile acid synthesis involves a deficiency in 7-hydroxylation due to a mutation in the gene for the microsomal oxysterol 7-hydroxylase enzyme, active in the acidic pathway for bile acid synthesis. The defect presents with severe cholestasis, cirrhosis, and liver synthetic failure. Diagnosis is established by fast atom bombardment ionization-mass spectrometry, which reveals elevated urinary excretion of sulfate and glycosulfate conjugates of

unsaturated monohydroxy-cholenoic acids, and an absence of primary bile acids. The biochemical findings are consistent with a deficiency in 7-hydroxylation, leading to the accumulation of hepatotoxic unsaturated monohydroxy bile acids.

Biliary atresia has been detected in 1/10,000-15,000 live births, idiopathic neonatal hepatitis in 1/5,000-10,000. Intrahepatic bile duct paucity appears much less commonly, in about 1/50,000-75,000 live births. Giant cell transformation of hepatocytes occurs frequently in infants with cholestasis and may occur in any form of neonatal liver injury. It is more frequent and more severe, however, in intrahepatic forms of cholestasis (neonatal hepatitis or intrahepatic bile duct paucity). Percutaneous liver biopsy is a valuable procedure in the evaluation of neonatal hepatobiliary diseases and provides the most reliable discriminatory evidence. Biliary atresia is characterized by bile ductular proliferation, the presence of bile plugs, and portal or perilobular edema and fibrosis, with the basic hepatic lobular architecture intact. In neonatal hepatitis, there is severe, diffuse hepatocellular disease, with distortion of lobular architecture, marked infiltration with inflammatory cells, and focal hepatocellular necrosis; the bile ductules show little alteration. Giant cell transformation is found in infants with either condition and has no diagnostic specificity.

Idiopathic neonatal hepatitis, which can occur in either a sporadic or a familial form, is a disease of unknown cause. These patients presumably are afflicted with a specific yet undefined metabolic or viral disease. In the past, patients with 1-antitrypsin deficiency were included in this category; after characterization of this specific metabolic disease, it is possible to define this subgroup of patients precisely. Infectious hepatitis in a neonate may be shown to be due to a specific virus, such as herpes simplex, enteroviruses, CMV, or, rarely, hepatitis B. This accounts for a small percentage of cases of neonatal hepatitis syndrome. Dubin-Johnson Syndrome. Dubin-Johnson syndrome is considered to be an autosomal recessive inherited defect in hepatocyte secretion of bilirubin glucuronide. The defect in hepatic excretory function is not limited to conjugated bilirubin excretion but also involves several organic anions normally excreted from the liver cell into bile. Absent function of multiple drug-resistant protein (MRP2), an adenosine triphosphate (ATP)-dependent canalicular transporter, is the responsible defect. Bile acid excretion is normal, and serum bile acid levels are normal. Urinary coproporphyrin excretion is normal in quantity; however, due to a defect in porphyrin excretion, coproporphyrin I constitutes 80% of the total. Coproporphyrin III is normally greater than 75% of the total. Cholangiography fails to visualize the biliary tract and roentgenography of the gallbladder is also abnormal. The liver cells contain black pigment similar to melanin. Rotor Syndrome. These patients have an additional deficiency in organic anion uptake. Total urinary coproporphyrin excretion is elevated, with a relative increase in the amount of the coproporphyrin I isomer. The gallbladder is normal by roentgenography, and liver cells contain

no black pigment. Sulfobromophthalein excretion is often abnormal.

LEAD POISONING After several weeks of lead accumulation and a BLL greater than 20 g/dL, increases in erythrocyte protoporphyrin (EP) levels greater than 35 g/dL may occur. An elevated EP level, not attributable to iron deficiency or recent inflammatory illness, is both an indicator of lead effect but also a useful test for assessing the success of the treatment; levels will begin to decrease a few weeks after successful interventions that reduce lead ingestion and increase lead excretion. Because EP is light sensitive, whole blood samples should be covered in aluminum foil (or equivalent) until analyzed. Because there is competition between lead and essential metals, it is reasonable to promote a healthy diet that is sufficient in calcium and iron content. The recommended daily intakes of these metals vary somewhat with age (see Chapter 40). In general, for children 1 yr of age and older a calcium intake of about 1 g/24 hr is sufficient. This is roughly the calcium content of a quart of milk (1200 mg/qt) or calcium-fortified orange juice. Calcium absorption is vitamin D dependent. Although milk is vitamin D fortified, other nutritional sources of calcium are not. A multivitamin containing vitamin D may be prescribed for those children not drinking sufficient milk or who have inadequate sunshine exposure. Iron requirements also vary with age: 6 mg/24 hr for infants to 12 mg/24 hr for adolescents. It is more difficult to eat enough iron-containing foods to attain this intake than to ingest calcium, and iron deficiency is commonly observed in young lead-poisoned children. For children identified biochemically as being iron deficient, therapeutic iron at a daily dose of 5-6 mg/kg for 3 mo is appropriate. Iron absorption is enhanced when ingested with ascorbic acid (citrus juices). Whether doses of calcium and/or iron greater than the daily requirements offer any additional benefits to lead-poisoned children is under investigation but cannot be recommended at this time. DMSA (succimer), CaNa2EDTA (versenate), BAL (dimercaprol), and penicillamine. DMSA and penicillamine can be given orally, whereas EDTA and BAL can only be administered parenterally. The choice of agent is guided by the severity of the lead poisoning, the effectiveness of the drug, and the ease of administration (Table 703-3). Children with BLLs between 44 and 70 g/dL should be treated with a single drug, preferably DMSA. Those with BLLs of 70 g/dL or greater require two-drug treatment: (1) CaNa2EDTA in combination with either DMSA or BAL for those without evidence of encephalopathy and (2) CaNa2EDTA and BAL for those with encephalopathy. Data on the combined treatment with CaNa2EDTA and DMSA for children with BLLs greater than 100 g/dL are very limited. Drug-related toxicities are generally minor and reversible. These include gastrointestinal distress, transient elevations in transaminase values, active urinary

sediment, and neutropenia. The frequencies of these types of events are lowest for CaNa2EDTA and DMSA and highest for BAL and penicillamine. Harrison 17th edition chelation is recommended with oral DMSA (succimer); if acutely toxic, hospitalization and IV or IM chelation with edentate calcium disodium (CaEDTA) may be required, with the addition of dimercaprol to prevent worsening of encephalopathy. Correction of dietary deficiencies in iron, calcium, magnesium, and zinc will lower lead absorption and may also improve toxicity. Vitamin C is a weak but natural chelating agent

Class Summary
Chelating agents are the criterion standards for the treatment of patients with lead poisoning according to the BLL mentioned above. These agents bind to lead and promote its excretion. Patients receiving chelation therapy must be closely monitored because of the agents' potential toxicities.
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Dimercaprol (BAL in oil)

First developed as an antidote for lewisite toxicity. Water soluble and rapidly crosses the blood-brain barrier. Forms a nonpolar compound with lead that is excreted in bile and urine. DOC in patients with acute lead encephalopathy, in whom first dose is given and then the second dose is given combined with calcium EDTA after a 4-h interval.
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Edetate calcium disodium (Versenate)

Decreases blood lead concentration, reverses hematologic effects of lead, and enhances excretion of lead in urine.
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Succimer (Chemet)
Dimercaptosuccinic acid (DMSA), a water-soluble analog of dimercaprol. Causes rapid decline in lead level and replenishes many of sulfhydryl-dependent enzymes. In absence of encephalopathy, patients may be treated with DMSA.

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D-penicillamine (Cuprimine, Depen)

Also known as D-dimethyl cysteine. Offers alternative for PO treatment of lead poisoning. Not FDA approved for use in lead poisoning, but has been in use for >20 y.

Vitamin E and necrotizing enterocolitis.

Finer NN, Peters KL, Hayek Z, Merkel CL.

Abstract
Although vitamin E has been shown to reduce the incidence of severe sequelae from retrolental fibroplasia, there have been recent suggestions that its use may be associated with an increased incidence of necrotizing enterocolitis (NEC). A review was made of experience with vitamin E, both intramuscular and oral, and NEC over a 4 1/2-year period. Of 418 infants of birth weight less than 1,500 g admitted during this period, 28/209 infants who had received vitamin E had definite NEC (13.4%) compared with 12/209 who had not received vitamin E (5.74%, chi 2 = 7.07, P = . 008). For infants of birth weight less than 1,250 g, 16/103 infants who received vitamin E developed NEC v 1/159 who had not (chi 2 = 21.1, P less than .001); the incidence of NEC was not significantly different between the two groups for infants with birth weight between 1,250 to 1,500 g. The early mortality (less than seven days) for infants with birth weight of 1,500 g or less was significantly greater for those who had not received vitamin E (43.5% v 13.8%, chi 2 = 44.9, P less than .001), most probably a reflection of the omission of this drug for the most critically ill infants in this retrospective review. The incidence of NEC was not different for infants with birth weight of 1,500 g or less who received intramuscular vitamin E compared with control infants from the same period. For those infants for whom serum tocopherol levels

Q. a seven year old asymptomatic girl with is found to have persistent hypertension. there is no significant history and urine examination is normal. which of the following is the most likely cause? essential hypertension or renovascular HTN Ans. renovascular/renal cause. Urine examination can be normal in renal causes. SD 2WQXCDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDDF

Q.all of the follwing factors are associted with a substantially greater risk of developing epilepsy after febrile seizures, except? a. complex febrile seizures b.early age of onset c. developmental anomilies d. positive history of epilepsy Ans. None of the above Although children with simple febrile seizures are at no greater risk of later epilepsy than the general population, some factors are associated with increased risk. These include the presence of atypical/complex features of the seizure or postictal period, a positive family history of epilepsy, an initial febrile seizure before 9 mo of age, delayed developmental milestones, or a pre-existing neurologic disorder. The incidence of epilepsy is approximately 9% when several risk factors are present, compared with an incidence of 1% in children who have febrile convulsions and no risk factors. Q. all of the following are good prognostic factors for childhood ALL except? a. t(12;21) b. pre B cell ALL c. female sex d. hyperdiploidy Ans. b. pre B cell ALL
PreB-ALLs include subtypes with a favorable prognosis, such as cases bearing the t(12;21)(p12;q22) or hyperdiploidy with greater than 50 chromosomes, and subtypes with an unfavorable prognosis, such as cases bearing the t(9;22)(q34;q11), the t (1;19)(q23;p13), or the t(4;11)(q21;q23).

Q.which of the following is the principle mode of heat exchange in an infant incubator? a. radiation b. evaporation c. convection d. conduction Ans. c. convection (incubator will always minimize heat loss) heat is lost by radiation and gained by convection........... Q. at what gestation age does the switchover from fetal to adult Hb synthesis begin? 30 or 36 wks Ans. 30 weeks Q. a child with perepheral circulatory failiure. the arterial pH is 7.0 , pCO2 of 15mmHg , pO2 76mm Hg. what of the following will be theimmediate therapy? bolus of ringer lactate or sodium bicarbonate infusion... Ans. bolus f ringer lactate. In a case of circulatory failure (Shock) with metabolic acidosis [present case], the most appropriate immediate treatment is to restore the intravascular volume by ringer lactate (fluid of choice for diarrhea) or normal saline. Q.one yr old male child presented with poor urinary stream since birth..the investigation of choice for evaluation..? a. VCUG b.USG bladder

c, IVU d. uroflowmetry Ans. a. VCUG. It is the investigation of choice for Dx of Posterior Uretheral valves which is the most common cause of severe obstructive uropathy in children. Poor/thin urinary stream is an imp. Symptom of Posterior Uretheral valves. Q.a 10 yr old child with headache,vomiting , gait instabilty and diplopia.On examination he had papillodema and gait ataxia. the most probable dx is. a.hydrocephalus b.brain stem tumor c. suprasellar tumor d. midline post. fossaa tumor Ans. d. midline post. fossaa tumor. It presents with raised ICT, cerebellar signs (gait ataxic), and cranial nerve palsies (diplopia). Q.failiure to pass meconium within 48hrs of birth in a newborn with no obvios external abnormality should lead to suspicion of ? a. anal atresia b. congenital pouch colon c. congenital aganglionosis d. meconium ileus Ans. c. congenital aganglionosis. It is the most common cause of delayed passage of meconium. Q.a neonate develops encephalitis withput any skin lesions. Most probable causative organism is? a. HSV-I b.HSV-II c.meningococci d.streptococci Ans. b.HSV-II (the common cause of genital lesions in mother from which neonate will acquire infection) Q.all of the following are ass with proximal muscle weakness except? a. SMA b. duchenes muscle dystrophy c. polio d. myotonic dystrophy both SMA and muotinic dystrophy have distal involvement. SMA is a motor neuron disease so it manifests only with muscle weakness and huporeflexia. Ans. d. myotonic dystrophy. All can cause proximal muscle weakness. But in myotonic dystrophy initially distal wasting occurs & proximal inv. Occurs very late.

Q.onle females are affected in ? a. schies syndrome b. hunter syndrome c. hurlers syndrome d. Gauchers disease Ans. This question is wrongly framed. Because a,c,d are AR diseases and occurs in both sexes. However option b is XLR & occurs in males. 1. Failure to initiate and maintain spontaneous respiration following birth is called a) birth asphyxia b) RDS c) resp. Failure Ans. a) birth asphyxia Because of birth/perinatal asphyxia, hypoxic/ischemic injury to brain can occur which results in failure of initiation and maintenance of spontaneous respiration. 2. Foetal length affected if mother has undernutrition during a)1st trimester b)2nd trimester c)3rd trimester d)any time during pregnancy Ans. a)1st trimester Fetal length corresponds with head circumference. So both fetal length & head circumference are affected only if insult to the fetus occurs in early pregnancy i.e. 1st trimester. Late pregnancy insult will affect only weight. 3. Administration of glucose solution is prescribed for all except a)neonate b)infant of diabetic mother c)h/o unconsciousnes Ans. a)neonate Unexplained Unconscious patient regimen is called coma cocktail which consists of Injection vitamin B1, i.v. glucose and naloxone. Infant of diabetic mother can also require i.v. glucose b/o high risk of hypoglycemia. A neonate should be breast fed (not to give i.v. glucose unnecessarily) 4. A 50 hr old breast fed ful term 3.1kg boy presents wid jaundice. physical examination normal. Total bilirubin is 11mg% n conjugated is 0.4mg%. Correct treatment a)continue breast feed n review after 48hr B) stop breast feed n review aftr 24hr Ans. continue breast feed n review after 48hr. This bilirubin value at 50 hr is in physiological range for a term baby (not in range of phototherapy). At this age (between 48 hr to 72 hr) cutoff to start phototherapy in a term baby is >15 mg/dL 5. Breast milk is known transmit a)tuberculosis b)CMV c)varicella

d)rubella Ans. CMV 6. A child presents wid diarhoea n peripheral circulatory failure. Arterial ph is 7,pco2 15mmhg,po2 76. Wat is most appropriate therapy a) NaHCo3 infusion b) bolus f ringer lactate c)5% dextrose Ans. b) bolus f ringer lactate. In a case of hypovolemic circulatory failure (Shock) with metabolic acidosis [present case], the most appropriate treatment is to restore the intravascular volume by ringer lactate (fluid of choice for diarrhea) or normal saline.

1.question already discused in class,2 wk old breast fed baby with freq vomiting and ftt.features of mod dehyderation.NA+122.K+6.1. blood urea-44 and creatinine-.6,what is diagniff 4 a a.21OH def b.ATN as told by u dat rise in creat has to be before k+rise which is not in dis case but in all d books i had see , givin nelson referance dey hav said dat values of creat and urea are diff 4 adults and in newborn babis and infants.givin diff vaues dey say dat urea and crat are high 4 dis age and so ans is ATN.plz guide.... Ans. this ques answer is given wrong in majority of MCQ text books. There is no doubt about this ques. ans. is 21OH def. Ans can be early ATN only if potasium value is given normal 2.12 year old boy wid severe vomit ad diarrohea bout 2 hospital of severe oliguria.he was given iv fluids and furesemide without any diuresis .bloood chemistry urea.120,creat-4.most likely diagnosis.... a.prerenal azotemia b.HUS c.AGN.d.ATN ans-b Ans. Although This ques statement is incomplete, but if u have to answer with this statement only, then ans. is ATN (B. urea/creatinine <50). If patients develop oliguria & azotemia during or immediately after the episode of diarrhea with BUN/creatinine ratio >20 or B. urea/creatinine >50 then likely dx is Prerenal azotemia. If patients develop oliguria & azotemia during or immediately after the episode of diarrhea with BUN/creatinine ratio <20 or B. urea/creatinine <50 then likely dx is Acute Tubular necrosis (ATN). If patients develop oliguria & azotemia after a gap of >/= 5 days of diarrhea, then likely dx is Hemolytic Uremic Syndrome. 3.1.5 kg child born at 32 weeks by LSCS wid mod respiratory diffR.R.70/min .which is management a.CPAP b.warm oxygen c. surfactant and ventilation d.mech ventilaton ans.c why not a.
Ans. This patient is most likely suffering from NRDS/HMD. For NRDS/HMD pts, the first line of treatment is nasal CPAP without surfactant, because nasal CPAP is : 1. less invasive procedure so less risk of nosocomial infection 2. less risk of barotrauma with CPAP as compared to mechanical ventilation. Surfactant can not be given with nasal CPAP, beacause to give surfactant you have to do endotracheal intubation. A baby starts producing his own surfactant by 3rd or 4th day of life. So if he is maintaing oxygenation on nasal CPAP, don't go for more invasive procedure i.e intubation 7 mechanical ventilation. Indication of mechanical ventilation in NRDS is when a baby doesn't maintain normal oxygenation or his work of breathing remains significant even after nasal CPAP. This indicates failure of nasal CPAP so you have to step up the treatment i.e. mechanical ventilation with surfactant.

4kg baby born to D.M.mother with lethargy ....a.reasses aftr 2 hrs,b.give 10%dextrose i.v. c.start oral feed ans is c. why nt b Ans. b.give 10%dextrose i.v. This patient is most likely having hypoglycemia which is symptomatic. For symptomatic (lethargy, seizures) hypoglycemia rx of choice is always i.v. glucose. For asymptomatic hypoglycemia rx of choice is oral feed or oral 10% dextrose. 5.in von willebrand disease ..Fac 8c qty is dec or nt,few books say its qty doesnt dec its functional activity dec while few like DR,bhatiya essence says dat it is 10-40.... harrison says about 80% which is normal Ans. Factor 8 levles can be low in vWD because a small fraction factor 8 is bound to vWF in blood & this fraction becomes low in vWD. But usually the levels of factor 8 don't become less than 40% (i.e. in hemophoilia range)
1, is the natural surfactant(surbenta bovine) given thruogh intrathecally like synthetic surfactant? And which is the best natural or synthetic? Ans. all surfactants (natural & synthetic) are given through endotracheal/intratracheal route & not intrathecally. Natural is better because more risk of pneumothorax with synthetic. 2,what is the indication of the mechanical ventilation( preffered simv)+surfactant over CPAP+SURFACTANT? Ans. For NRDS/HMD pts, the first line of treatment is nasal CPAP without surfactant, because nasal CPAP is : 1. less invasive procedure so less risk of nosocomial infection 2. less risk of barotrauma with CPAP as compared to mechanical ventilation. Surfactant can not be given with nasal CPAP, beacause to give surfactant you have to do endotracheal intubation. A baby starts producing his own surfactant by 3rd or 4th day of life. So if he is maintaing oxygenation on nasal CPAP, don't go for more invasive procedure i.e intubation 7 mechanical ventilation. Indication of mechanical ventilation in NRDS is when a baby doesn't maintain normal oxygenation or his work of breathing remains significant even after nasal CPAP. This indicates failure of nasal CPAP so you have to step up the treatment i.e. mechanical ventilation with surfactant. 3, What is the ideal time for cord clamping according to the recent guideline immeditely or delayed? Ans. Immediate

Q1..sir,plz clear me with the most common causes of meningitis in different age gps? Ans. <2mo: Group B streptococcus (Strept. agalactae) >2mo: Pneumoccoccal 2mo-3yr in India: H. influenzae Q2..gestational age of hb. switchover 4m fetal to adult? Ans. 30-32 wks Q3wen does urine conc capacity of baby becum equal to adults? a)1 year b)18 months Ans. 18 months Q4..a 6 months old girl is having rec. UTI.USG abd shoows b/l hydronephrpsis. MCU shows grade 4 VUR...treatment of choice is..

ans given is ureteric transplantation...and d options also have prophylactic antibiotics..AIPGME 2002 Ans. prophylactic antibiotics Q5..a pt presents with LVH and pulmonary comlications.ECG shows left axis deviation...diagnosis is.. ans given is Tricuspid atresia..why cant the ans be VSD Ans. VSD. But if compromised pulmonary blood flow is given in place of pulmonary complications then ans. will be Tricuspid atresia. Q6wat shd be measured in newborn with hyperbil.. a)total bil b)total and direct bil Ans. total and direct bil. To differentiate cholestasis from indirect hyperbilirubinemia 1.question already discused in class,2 wk old breast fed baby with freq vomiting and ftt.features of mod dehyderation.NA+122.K+6.1. blood urea-44 and creatinine-.6,what is diagniff 4 a a.21OH def b.ATN as told by u dat rise in creat has to be before k+rise which is not in dis case but in all d books i had see , givin nelson referance dey hav said dat values of creat and urea are diff 4 adults and in newborn babis and infants.givin diff vaues dey say dat urea and crat are high 4 dis age and so ans is ATN.plz guide.... Ans. this reference doesnt exist in nelson. Yes values of creat and urea are diff 4 adults and in newborn babis and infants. Creatinine 0.6 is upper cut off of normal in newborns. Ans is 21OH def. no doubt at all. 2.12 year old boy wid severe vomit ad diarrohea bout 2 hospital of severe oliguria.he was given iv fluids and furesemide without any diuresis .bloood chemistry urea.120,creat-4.most likely diagnosis.... a.prerenal azotemia b.HUS c.AGN.d.ATN ans-b Kindly complete the question words, and also mention oliguria occured after how many days of diarrhea if given in ques. 3.1.5 kg child born at 32 weeks by LSCS wid mod respiratory diffR.R.70/min .which is management a.CPAP b.warm oxygen c. surfactant and ventilation d.mech ventilaton ans.c why not a. Ans. a.CPAP 4kg baby born to D.M.mother with lethargy ....a.reasses aftr 2 hrs,b.give 10%dextrose i.v. c.start oral feed ans is c. why nt b 5.in von willebrand disease ..Fac 8c qty is dec or nt,few books say its qty doesnt dec its functional activity dec while few like DR,bhatiya essence says dat it is 10-40.... harrison says about 80% which is normal Ans.